Intragenic deletions of the dystrophin-encoding and muscular dystrophy-associated DMD gene have been recently described in gastrointestinal stromal tumor (GIST), rhabdomyosarcoma (RMS) and leiomyosarcoma (LMS). W...
Authors: Maria A Pantaleo, Annalisa Astolfi, Milena Urbini, Fabio Fuligni, Maristella Saponara, Margherita Nannini, Cristian Lolli, Valentina Indio, Donatella Santini, Giorgio Ercolani, Giovanni Brandi, Antonio D Pinna and Guido Biasco
Dermatofibrosarcoma Protuberans (DFSP) is a rare skin tumor, characterized by frequent local recurrence but is seldom metastatic. It is histologically characterized by storiform arrangement of spindle cells. C...
Authors: Mashaal Dhir, David G Crockett, Todd M Stevens, Peter T Silberstein, William J Hunter and Jason M Foster
We retrospectively reviewed data from nine pre-treated metastatic desmoplastic small round cell tumour (DSRCT) patients who received pazopanib.
Authors: Anna Maria Frezza, Charlotte Benson, Ian R Judson, Saskia Litiere, Sandrine Marreaud, Stefan Sleijfer, Jean-Yves Blay, Raz Dewji, Cyril Fisher, Winette van der Graaf and Larry Hayward
Follicular dendritic cell sarcoma is a rare malignancy arising from follicular dendritic cells, which form a meshwork within lymphoid follicles. Traditional treatment of metastatic disease with CHOP chemothera...
Aromatase inhibitors (AIs) have not been used consistently as part of the management of hormone receptor positive uterine leiomyosarcomas (ULMS). As a result, the published data regarding the efficacy of AIs i...
Authors: Eirini Thanopoulou, Khin Thway, Komel Khabra and Ian Judson
This is the report of the 2nd Joint ENCCA/EuroSARC European Bone Sarcoma Network Meeting held in Leiden, The Netherlands, on 26-27 September 2013, bringing together preclinical and clinical investigators on bone ...
Authors: Jakob K Anninga, Anne-Marie Cleton-Jansen, Bass Hassan, M Fernanda Amary, Daniel Baumhoer, Jean-Yves Blay, Laurence Brugieres, Stefano Ferrari, Heribert Jürgens, Beate Kempf-Bielack, Heinrich Kovar, Ola Myklebost, Michaela Nathrath, Piero Picci, Peter Riegman, Marco W Schilham…
Desmoplastic small round cell tumour (DSRCT) is a rare sarcoma typically affecting young males and usually widely metastatic at presentation. Despite multimodal treatment approaches, the prognosis for DSRCT is...
Authors: Anna Maria Frezza, Jeremy S Whelan and Palma Dileo
Serum protein acidic and rich in cysteine (SPARC) is a matricellular secreted glycoprotein that performs several cellular functions and has been implicated in tumorigenesis in a variety of tumor types. The che...
Authors: Sherif S Morgan, Raymond B Nagle and Lee D Cranmer
Epstein-Barr virus (EBV)-associated post-transplant smooth muscle tumours (PTSMT), are rare complications following organ/stem cell transplantation. Despite the mainly benign behaviour of PTSMT, alternative th...
Authors: Danny Jonigk, Nicole Izykowski, Lavinia Maegel, Eileen Schormann, Britta Ludewig, Hans Kreipe and Kais Hussein
Extraskeletal myxoid chondrosarcoma (EMC) is a rare subgroup within soft tissue sarcomas. Its sensitivity to chemotherapy is reported to be low.
Authors: Silvia Stacchiotti, Gian Paolo Dagrada, Roberta Sanfilippo, Tiziana Negri, Isabella Vittimberga, Stefano Ferrari, Federica Grosso, Gaetano Apice, Marco Tricomi, Chiara Colombo, Alessandro Gronchi, Angelo P Dei Tos, Silvana Pilotti and Paolo G Casali
Data suggests that males experience less toxicity and poorer survival than females treated for Ewing’s sarcoma. We instituted an intra-patient dose escalation (DE) policy with Vincristine/Doxorubicin/Cyclophos...
Authors: Jeremy Lewin, Samantha Wieringa, Marnie Collins, Jayesh Desai, Lisa Orme, Senthil Lingaratnam and David M Thomas
Desmoplastic small round cell tumour (DSRCT) is a rare but frequently fatal sarcoma, and many of its characteristics still require further clarification.
Authors: Han Hsi Wong, Helen M Hatcher, Charlotte Benson, Omar Al-Muderis, Gail Horan, Cyril Fisher, Helena M Earl and Ian Judson
Desmoid tumours/aggressive fibromatosis (DT/AF) are infrequent soft-tissue neoplasms. They usually behave as indolent diseases. However, they may grow locally infiltrating or compressing adjacent structures. T...
Authors: Juan Martin-Liberal, Charlotte Benson, Heather McCarty, Khin Thway, Christina Messiou and Ian Judson
Gastrointestinal stromal tumors are rare soft tissue sarcomas that typically develop from mesenchymal cells with acquired gain-in-function mutations in KIT or PDGFRA oncogenes. These somatic mutations have been w...
Authors: Katie M O’Brien, Irene Orlow, Cristina R Antonescu, Karla Ballman, Linda McCall, Ronald DeMatteo and Lawrence S Engel
The study aimed to examine attitudes of individuals diagnosed with sarcoma and their family members towards genetics, genomic research and incidental information arising as a result of participating in genetic...
Authors: Mary-Anne Young, Amy Herlihy, Gillian Mitchell, David M Thomas, Mandy Ballinger, Kathy Tucker, Craig R Lewis, Susan Neuhaus and Jane Halliday
MSCs are hypothesized to potentially give rise to sarcomas after transformation and therefore serve as a good model to study sarcomagenesis. Both spontaneous and induced transformation of MSCs have been report...
Authors: Wei Xiao, Alexander B Mohseny, Pancras C W Hogendoorn and Anne-Marie Cleton-Jansen
Epstein-Barr virus (EBV)-associated post-transplant smooth muscle tumours (PTSMT) are rare complications. In our previous molecular analysis, we have evaluated the expression of regulatory microRNA which are k...
Authors: Danny Jonigk, Nicole Izykowski, Lavinia Maegel, Eileen Schormann, Britta Maecker-Kolhoff, Florian Laenger, Hans Kreipe and Kais Hussein
Pigmented villonodular synovitis (PVNS) is a rare locally aggressive tumor. PVNS is characterized in most cases by a specific t(1;2) translocation, which fuses the colony stimulating factor-1 (CSF1) gene to the c...
Authors: Silvia Stacchiotti, Flavio Crippa, Antonella Messina, Silvana Pilotti, Alessandro Gronchi, Jean Y Blay and Paolo G Casali
Patients with advanced solitary fibrous tumors (SFTs) have a poor prognosis; treatment options for recurrent disease are particularly limited. Several novel targeted agents have recently shown promise against ...
Authors: Min S Park, Vinod Ravi, Anthony Conley, Shreyaskumar R Patel, Jonathan C Trent, Dina C Lev, Alexander J Lazar, Wei-Lien Wang, Robert S Benjamin and Dejka M Araujo
We surveyed the natural history of bone metastases in patients affected by soft tissue sarcoma (STS).
Authors: Bruno Vincenzi, Anna Maria Frezza, Gaia Schiavon, Daniele Santini, Palma Dileo, Marianna Silletta, Delia Delisi, Francesco Bertoldo, Giuseppe Badalamenti, Giacomo Giulio Baldi, Stefania Zovato, Rossana Berardi, Marco Tucci, Franco Silvestris, Angelo Paolo Dei Tos, Roberto Tirabosco…
DFSP is a locally invasive, slow-growing tumor of the subcutaneous tissue that rarely metastasizes but recurs frequently after surgical excision. We report herein a case of highly recurrent, locally invasive D...
Authors: Francois G Kamar, Victor F Kairouz and Alain N Sabri
We explore the pattern of late recurrence (LR) in solitary fibrous tumor (SFT), focusing on histopathologic characteristics, clinical presentation and patients (pts) outcome.
Authors: Giacomo G Baldi, Silvia Stacchiotti, Valentina Mauro, Angelo P Dei Tos, Alessandro Gronchi, Ugo Pastorino, Leonardo Duranti, Salvatore Provenzano, Andrea Marrari, Michela Libertini, Silvana Pilotti and Paolo G Casali
The systemic treatment of malignant endometrial stromal tumors (EST) is not well established. A few reports describe objective responses to imatinib, which suggest a novel therapeutic strategy for these tumors...
Authors: Ruth Sardinha, Teresa Hernández, Susana Fraile, Francesc Tresserra, August Vidal, Maria Carmén Gómez, Aurora Astudillo, Nieves Hernández, Javier Saenz de Santamaría, Jaume Ordi, Luis Gonçalves, Rafael Ramos, Carmen Balañá and Enrique de Álava
Ewing sarcoma (ES) is a high-grade malignant primary round cell tumour of bone in which there is commonly extension into extraosseous soft tissues at the time of diagnosis. This report details the clinical, ra...
Authors: Takeshi G Kashima, Nimali G Gamage, Uta Dirksen, Christopher L M H Gibbons, Simon J Ostlere and Nicholas A Athanasou
Trabectedin and thioglitazones have been documented to induce adipocytic maturation in myxoid liposarcoma; we have noted this in our experience as well. Intriguingly, we have also encountered this same phenome...
Authors: Wei-Lien Wang, Daniela Katz, Dejka M Araujo, Vinod Ravi, Joseph A Ludwig, Jonathan C Trent, Shreyaskumar R Patel, Patrick P Lin, Ashleigh Guadagnolo, Dolores Lòpez-Terrada, Angelo Paola dei Tos, Valerie O Lewis, Dina Lev, Raphael E Pollock, Gunar K Zagars, Robert S Benjamin…
Chemokine receptor CXCR4, together with its ligand CXCL12, plays critical roles in cancer progression, including growth, metastasis and angiogenesis. Ewing sarcoma is a sarcoma with poor prognosis despite curr...
Authors: Dagmar Berghuis, Marco W Schilham, Susy J Santos, Suvi Savola, Helen J Knowles, Uta Dirksen, Karl-Ludwig Schaefer, Jukka Vakkila, Pancras CW Hogendoorn and Arjan C Lankester
Long-term continuous imatinib is recommended for adult patients with unresectable and/or metastatic KIT+ gastrointestinal stromal tumors (GIST) as long as the patient continues to benefit. In the adjuvant sett...
Extraskeletal myxoid chondrosarcoma (EMCS) is a rare soft tissue sarcoma of uncertain differentiation, characterized in most cases by a translocation that results in the fusion protein EWSR1-CHN (the latter even ...
Authors: Silvia Stacchiotti, Gian Paolo Dagrada, Carlo Morosi, Tiziana Negri, Antonella Romanini, Silvana Pilotti, Alessandro Gronchi and Paolo G Casali
The current AJCC version 7 staging system is a lugubrious means to classify gastrointestinal stromal tumors with respect to their risk and need for adjuvant systemic therapy. The rule of fives, suggested here,...
Sarcomas are neoplastic malignancies that typically arise in tissues of mesenchymal origin. The identification of novel molecular mechanisms leading to sarcoma formation and the establishment of new therapies ...
Osteosarcoma is the most common form of bone cancer. Pivotal insight into the genes involved in human osteosarcoma has been provided by the study of rare familial cancer predisposition syndromes. Three kindred...
Authors: Alvin JM Ng, Anthony J Mutsaers, Emma K Baker and Carl R Walkley
Sarcomas were one of the first solid cancers to be linked to ionizing radiation exposure. We reviewed the current evidence on this relationship, focusing particularly on the studies that had individual estimat...
Authors: Amy Berrington de Gonzalez, Alina Kutsenko and Preetha Rajaraman
Malignant peripheral nerve sheath tumours (MPNST) are rare tumours known to occur at high frequency in neurofibromatosis 1 (NF1), but may also occur in other cancer prone syndromes.
Authors: D Gareth R Evans, Susan M Huson and Jillian M Birch
The discovery of underlying molecular genetic abnormalities in gastrointestinal stromal tumors (GISTs) such as activating mutations in the tyrosine kinase genes, KIT and platelet derived growth factor receptor-al...
Children diagnosed with the hereditary form of retinoblastoma (Rb), a rare eye cancer caused by a germline mutation in the RB1 tumor suppressor gene, have excellent survival, but face an increased risk of bone an...
Authors: Ruth A Kleinerman, Sara J Schonfeld and Margaret A Tucker
Sarcomas account for over 20% of all pediatric solid malignant cancers and less than 1% of all adult solid malignant cancers. The vast majority of diagnosed sarcomas will be soft tissue sarcomas, while maligna...
Authors: Zachary Burningham, Mia Hashibe, Logan Spector and Joshua D Schiffman
To evaluate tolerability and maintenance of dose intensity of 2 weekly treatment with vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide, etoposide (VDC/IE) in patients with advanced small ...
Authors: Jeremy Whelan, Atia Khan, Anand Sharma, Christian Rothermundt, Palma Dileo, Maria Michelagnoli, Beatrice Seddon and Sandra Strausss
Recombinant erythropoietin for the anaemia of patients with advanced Gastrointestinal Stromal Tumours (GIST) receiving imatinib : an active agent only in non progressive patients.
Authors: Florence Duffaud, Caroline Even, Isabelle Ray-Coquard, Emmanuelle Bompas, Thanh Khoa-Huynh, Sebastien Salas, Philippe Cassier, Armelle Dufresne, Sylvie Bonvalot, Francoise Ducimetiere, Axel Le Cesne and Jean-Yves Blay
Phenol is widely used for years as local adjuvant treatment for bone tumours. Despite its use for a long time, no information is available about the local concentration of phenol that is achieved in an individ...
Authors: Suzan HM Verdegaal, Jan den Hartigh, Pancras CW Hogendoorn, Hugo FG Brouwers and Antonie HM Taminiau
Osteosarcoma is the most common primary high-grade bone tumor in both adolescents and children. Early tumor detection is key to ensuring effective treatment. Serum marker discovery and validation for pediatric...
Authors: Yulia A Savitskaya, Genaro Rico-Martínez, Luis Miguel Linares-González, Ernesto Andrés Delgado-Cedillo, René Téllez-Gastelum, Alfonso Benito Alfaro-Rodríguez, Antonio Redón-Tavera and José Clemente Ibarra-Ponce de León
Ewing sarcoma patients have a poor prognosis despite multimodal therapy. Integration of combination immunotherapeutic strategies into first-/second-line regimens represents promising treatment options, particu...
Authors: Dagmar Berghuis, Marco W Schilham, Hanneke I Vos, Susy J Santos, Stephan Kloess, Emilie P Buddingh', R Maarten Egeler, Pancras CW Hogendoorn and Arjan C Lankester
Ewing Sarcoma is the second most common primary bone sarcoma with 900 new diagnoses per year in Europe (EU27). It has a poor survival rate in the face of metastatic disease, with no more than 10% survival of t...
Authors: Annmeik M van Maldegem, Pancras CW Hogendoorn and Andrew B Hassan
Ewing sarcoma/PNET is managed with treatment paradigms involving combinations of chemotherapy, surgery, and sometimes radiation. Although the 5-year survival rate of non-metastatic disease approaches 70%, thos...
High grade primary bone sarcomas are rare cancers that affect mostly children and young adults. Osteosarcoma and Ewing sarcoma are the most common histological subtypes in this age group, with current multimod...
Authors: Annemiek M van Maldegem, Aparna Bhosale, Hans J Gelderblom, Pancras CW Hogendoorn and Andrew B Hassan
Dermatofibrosarcoma protuberans (DFSP) is a relatively common soft-tissue tumor. A more aggressive appearing fibrosarcoma may arise in DFSP, changing its biological behavior. CD34 and apolipoprotein-D are high...
Authors: Emanuela Palmerini, Marco Gambarotti, Eric L Staals, Licciana Zanella, Gabriela Sieberova, Alessandra Longhi, Marilena Cesari, Stefano Bonarelli, Piero Picci, Pietro Ruggieri, Marco Alberghini and Stefano Ferrari
Desmoplastic small round cell tumour is a rare malignant tumour with a male to female ratio of 4:1. It manifests mostly at serosal sites. Here we present a case of a 28-year-old male patient, who presented wit...
Authors: Vincent PM Cliteur, Károly Szuhai, Hans J Baelde, Jurriaan van Dam, Hans Gelderblom and Pancras CW Hogendoorn
Myxoid liposarcoma (MLS) is a soft tissue sarcoma with adipocytic differentiation characterized by a unique chromosome rearrangement, t(12;16)(q13;p11). The exact efficacy of chemotherapy in MLS has not been c...
Authors: Daniela Katz, Piyaporn Boonsirikamchai, Haeson Choi, Alexander J Lazar, Wei-Lein Wang, Lianchun Xiao, Min S Park, Vinod Ravi, Robert S Benjamin and Dejka M Araujo
Sarcomas are rare, often fatal malignancies of connective tissues that can occur in genetic predisposition syndromes or result from carcinogen exposure. Hodgkin's lymphoma (HL) is not known to contribute to an...
Authors: Megan E Downing, Gillian S Dite and Mandy L Ballinger
